Pericranial Flap After Reconstruction of Malignant Peripheral Schwannoma Transformed from Neurofibroma in a Patient with Neurofibromatosis Type 1

Abstract

 Malignant peripheral nerve sheath tumors (MPNSTs), which derive from Schwann cells, account for approximately 7% of malignant soft tissue tumors. More than half of MPNSTs are associated with neurofibromatosis type 1 (NF1). Herein, we report a patient with NF1 who experienced malignant transformation of a neurofibroma and was treated with pericranial flap reconstruction after repeated excisions. The patient was a 73-year-old woman who first noticed a cutaneous tumor in the frontal region when she was 58 years old. Following the initial excision, the tumor continued to recur, leading to a total of three excisions. The histopathologic diagnosis was neurofibroma in all instances. At the age of 73, the patient required another resection. Pathology results revealed MPNST with malignant transformation of the neurofibroma. After extensive subperiosteal resection and confirmation of negative margins, reconstruction using a pericranial flap and two-stage full-thickness skin grafting were performed with satisfactory results.